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Common and distinctive pathogenetic features of arteriovenous malformations in hereditary hemorrhagic telangiectasia 1 and hereditary hemorrhagic telangiectasia 2 animal models--brief report.
Garrido-Martin, Eva M; Nguyen, Ha-Long; Cunningham, Tyler A; Choe, Se-Woon; Jiang, Zhihua; Arthur, Helen M; Lee, Young-Jae; Oh, S Paul.
Afiliação
  • Garrido-Martin EM; From the Department of Physiology and Functional Genomics (E.M.G.-M., H.-L.N., T.A.C., S.-w.C., S.P.O.) and Department of Surgery (Z.J.), University of Florida, Gainesville; Department of Biomedical Engineering, Tongmyong University, Busan, Republic of Korea (S.-w.C.); Institute of Genetic Medicine,
  • Nguyen HL; From the Department of Physiology and Functional Genomics (E.M.G.-M., H.-L.N., T.A.C., S.-w.C., S.P.O.) and Department of Surgery (Z.J.), University of Florida, Gainesville; Department of Biomedical Engineering, Tongmyong University, Busan, Republic of Korea (S.-w.C.); Institute of Genetic Medicine,
  • Cunningham TA; From the Department of Physiology and Functional Genomics (E.M.G.-M., H.-L.N., T.A.C., S.-w.C., S.P.O.) and Department of Surgery (Z.J.), University of Florida, Gainesville; Department of Biomedical Engineering, Tongmyong University, Busan, Republic of Korea (S.-w.C.); Institute of Genetic Medicine,
  • Choe SW; From the Department of Physiology and Functional Genomics (E.M.G.-M., H.-L.N., T.A.C., S.-w.C., S.P.O.) and Department of Surgery (Z.J.), University of Florida, Gainesville; Department of Biomedical Engineering, Tongmyong University, Busan, Republic of Korea (S.-w.C.); Institute of Genetic Medicine,
  • Jiang Z; From the Department of Physiology and Functional Genomics (E.M.G.-M., H.-L.N., T.A.C., S.-w.C., S.P.O.) and Department of Surgery (Z.J.), University of Florida, Gainesville; Department of Biomedical Engineering, Tongmyong University, Busan, Republic of Korea (S.-w.C.); Institute of Genetic Medicine,
  • Arthur HM; From the Department of Physiology and Functional Genomics (E.M.G.-M., H.-L.N., T.A.C., S.-w.C., S.P.O.) and Department of Surgery (Z.J.), University of Florida, Gainesville; Department of Biomedical Engineering, Tongmyong University, Busan, Republic of Korea (S.-w.C.); Institute of Genetic Medicine,
  • Lee YJ; From the Department of Physiology and Functional Genomics (E.M.G.-M., H.-L.N., T.A.C., S.-w.C., S.P.O.) and Department of Surgery (Z.J.), University of Florida, Gainesville; Department of Biomedical Engineering, Tongmyong University, Busan, Republic of Korea (S.-w.C.); Institute of Genetic Medicine,
  • Oh SP; From the Department of Physiology and Functional Genomics (E.M.G.-M., H.-L.N., T.A.C., S.-w.C., S.P.O.) and Department of Surgery (Z.J.), University of Florida, Gainesville; Department of Biomedical Engineering, Tongmyong University, Busan, Republic of Korea (S.-w.C.); Institute of Genetic Medicine,
Arterioscler Thromb Vasc Biol ; 34(10): 2232-6, 2014 Oct.
Article em En | MEDLINE | ID: mdl-25082229
ABSTRACT

OBJECTIVE:

Hereditary hemorrhagic telangiectasia is a genetic disorder characterized by visceral and mucocutaneous arteriovenous malformations (AVMs). Clinically indistinguishable hereditary hemorrhagic telangiectasia 1 and hereditary hemorrhagic telangiectasia 2 are caused by mutations in ENG and ALK1, respectively. In this study, we have compared the development of visceral and mucocutaneous AVMs in adult stages between Eng- and Alk1-inducible knockout (iKO) models. APPROACH AND

RESULTS:

Eng or Alk1 were deleted from either vascular endothelial cells (ECs) or smooth muscle cells in adult stages using Scl-CreER and Myh11-CreER lines, respectively. Latex perfusion and intravital spectral imaging in a dorsal skinfold window chamber system were used to visualize remodeling vasculature during AVM formation. Global Eng deletion resulted in lethality with visceral AVMs and wound-induced skin AVMs. Deletion of Alk1 or Eng in ECs, but not in smooth muscle cells, resulted in wound-induced skin AVMs. Visceral AVMs were observed in EC-specific Alk1-iKO but not in Eng-iKO. Intravital spectral imaging revealed that Eng-iKO model exhibited more dynamic processes for AVM development when compared with Alk1-iKO model.

CONCLUSIONS:

Both Alk1- and Eng-deficient models require a secondary insult, such as wounding, and ECs are the primary cell type responsible for the pathogenesis. However, Alk1 but not Eng deletion in ECs results in visceral AVMs.
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Texto completo: 1 Base de dados: MEDLINE Assunto principal: Malformações Arteriovenosas / Telangiectasia Hemorrágica Hereditária Tipo de estudo: Prognostic_studies Limite: Animals Idioma: En Ano de publicação: 2014 Tipo de documento: Article
Texto completo
Imprimir
XML
PubMed Links
Buscar no Google

Texto completo: 1 Base de dados: MEDLINE Assunto principal: Malformações Arteriovenosas / Telangiectasia Hemorrágica Hereditária Tipo de estudo: Prognostic_studies Limite: Animals Idioma: En Ano de publicação: 2014 Tipo de documento: Article